Furudate Sadanori, Fujimura Taku, Hashimoto Akira, Aiba Setsuya
Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Case Rep Dermatol. 2011 May;3(2):113-7. doi: 10.1159/000329000. Epub 2011 May 20.
We describe a 50-year-old Japanese patient with dermatofibrosarcoma protuberans (DFSP) rapidly growing after bilateral adrenalectomy for Cushing's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg/dl. It is known that glucocorticoids decrease the transcriptions of the COL1A1 gene and the PDGFB gene, which is under the direct control of the COL1A1 gene in most DFSP. Therefore, the hypersecretion of glucocorticoids in Cushing's syndrome might suppress the development of DFSP. To the best of our knowledge, this is the first case of rapid growth of DFSP that may be associated with bilateral adrenalectomy for Cushing's syndrome.
我们描述了一名50岁的日本患者,其患有隆突性皮肤纤维肉瘤(DFSP),在因库欣综合征行双侧肾上腺切除术后迅速生长,该手术使血清皮质醇水平从17.1降至0.8mg/dl。已知糖皮质激素会降低COL1A1基因和PDGFB基因的转录,而在大多数DFSP中,PDGFB基因受COL1A1基因的直接调控。因此,库欣综合征中糖皮质激素的过度分泌可能会抑制DFSP的发展。据我们所知,这是首例DFSP迅速生长可能与因库欣综合征行双侧肾上腺切除术相关的病例。